Abstract
Purpose Because Hirayama disease is stereotyped as a self-limited disease in the absence of a definite pathology, we investigated the potential relationship between snake-eyes appearance (SEA) and Hirayama disease to bring a new perspective in the pathological process of Hirayama disease based on relevant radiological and clinical evidence. Methods A total of 30 cases observed SEA were selected from 293 patients with Hirayama disease to constitute the SEA group, and an equal number of cases were randomly selected from the remaining patients to form the non-SEA group. Cervical magnetic resonance imaging (MRI) was performed and subsequently used to measure the anteroposterior diameter and anterior shifting of the spinal cord. Additionally, clinical data, such as age, sex, duration of symptoms, symptoms, and signs, were collected and analyzed. Results Of 293 patients, 10.6% appeared with the SEA, which was mainly multisegmental (86.7%), particularly at the C5-6 segment (73.3%), and intense with a well-defined border (70.0%). The SEA group was an older population (p < 0.0001) with a longer duration (p < 0.0001) and a higher incidence of Hoffmann signs and knee hyperreflexia (p < 0.0001, p = 0.0038, respectively). The degree of spinal cord atrophy demonstrated a close association with the SEA, as it was significantly worse in the SEA group and SEA segment (p = 0.0008, p < 0.0001, respectively). The degree of spinal cord atrophy was positively related to both age and duration (p = 0.0095, p = 0.0176, respectively). Conclusions Confirmed as an irreversible lesion and an indication of poor prognosis, SEA appears during the late stage of Hirayama disease and is closely related to pyramidal signs and spinal cord atrophy.
Highlights
Hirayama disease is an insidious distal upper limb weakness and atrophy, which occurs mainly in young males [1]
Classical magnetic resonance imaging (MRI) findings of Hirayama disease include the following: (1) an anterior shifting of the spinal cord with forward compression and focal atrophy in the lower cervical cord; and (2) anterior shifting of the posterior dura and the resulting venous plexus dilatation appearing as a crescent or focal mass with flow voids in the enlarged posterior epidural space displaying strong contrast enhancement [2, 3]
We found that Hoffmann signs and knee hyperreflexia could appear in these patients, with the Snake-eyes appearance (SEA) on MRI, which is contrary to the conventional view that Hirayama disease is a self-limited disease in the absence of pyramidal signs [8]
Summary
Hirayama disease is an insidious distal upper limb weakness and atrophy, which occurs mainly in young males [1]. Classical magnetic resonance imaging (MRI) findings of Hirayama disease include the following: (1) an anterior shifting of the spinal cord with forward compression and focal atrophy in the lower cervical cord; and (2) anterior shifting of the posterior dura and the resulting venous plexus dilatation appearing as a crescent or focal mass with flow voids in the enlarged posterior epidural space displaying strong contrast enhancement [2, 3]. Snake-eyes appearance (SEA) is a unique radiological finding characterized as a symmetrical bilateral small highsignal-intensity lesion on an axial T2-weighted MRI and is named because of its similar appearance to the face of a snake (Figures 1(a) and 1(b)). There is an urgent necessity to better understand the relationship between Hirayama disease and SEA and to reconsider the pathological process of Hirayama disease based on relevant radiological and clinical evidence
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